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Periodic Limb Movement

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3041. Huntington's Disease

with HD it has been reported to be as common as 69%. [ ] HD then leads to progressive chorea, rigidity and dementia. It is frequently associated with seizures. Chorea is initially mild but may be severe and cause uncontrollable limb movements. As the disease progresses, chorea is gradually replaced by dystonia and Parkinsonian features. Dysarthria, dysphagia and abnormal eye movements are common. There may also be other movement disorders - eg, tics and myoclonus. HD patients can develop a wide array (...) variable. JHD (6% of all cases of HD) is defined as an age of onset of younger than 20 years. It causes Parkinsonian features, dystonia, pyramidal tract signs, dementia and epilepsy. Chorea is often mild and may be absent. Differential diagnosis Neuroacanthocytosis: a group of genetic conditions that are characterised by movement disorders and acanthocytosis (abnormally-shaped red blood cells). and other causes of . Other causes of . Investigations MRI and CT scans in moderate-to-severe HD show a loss

2008 Mentor

3042. Hand Arm Vibration Syndrome

Guidelines. You may find the article more useful, or one of our other . In this article In This Article Hand-arm Vibration Syndrome In this article Synonyms: HAVS, vibration white finger, VWF Prolonged exposure to hand-arm vibration may cause a potentially disabling chronic disorder of the upper limbs, which is called the hand-arm vibration syndrome (HAVS). As well as vascular and neurological injury, HAVS may also involve damage to bones, joints, muscles and tendons in the upper limbs. There may also (...) (little finger) in each hand at two frequencies. The thermal aesthesiometry test, which assesses the thermal receptors' threshold. Readings are again taken from the median nerve and the ulnar nerve. The Purdue Pegboard test, which is a measure of dexterity and detects loss of fine movement to both hands. The grip force measurement test, which measures the grip strength of both hands. This test is not scored but is a useful indication of vibration damage to muscles of the hand and forearm. In addition

2008 Mentor

3043. Managing Epilepsy in Primary Care

, which lower the seizure threshold. It is common for seizure frequency to vary throughout the menstrual cycle. In ovulatory cycles, peaks occur around the time of ovulation and in the few days before menstruation. In anovulatory cycles, there is an increase in seizures during the second half of the menstrual cycle. [ ] Possible seizure-related symptoms include: Sudden falls. Involuntary jerky movements of limbs whilst awake. Blank spells. Unexplained incontinence of urine with loss of awareness (...) , or in sleep. Odd events occurring in sleep - eg, fall from bed, jerky movements, automatisms. Episodes of confused behaviour with impaired awareness. Possible simple focal seizures. Epigastric fullness sensation. Déjà vu. Premonition. Fear. Elation, depression. Depersonalisation, derealisation. Inability to understand or express language (written or spoken). Loss of memory, disorientation. Olfactory, gustatory, visual, auditory hallucinations. Focal motor or somatosensory deficit, or positive symptoms

2008 Mentor

3044. Multiple Sclerosis

cord. In this way movement and sensation may be impaired. The causes of MS are not completely understood but the autoimmune process appears to be caused both by genetic and environmental factors - eg, viral infections in early life. Minor viral infections frequently precipitate relapses. There are different patterns of MS: Relapsing-remitting MS : symptoms come and go. Periods of good health or remission are followed by sudden symptoms or relapses (80% of people at onset). Secondary progressive MS (...) : follows on from relapsing-remitting MS. There are gradually more or worsening symptoms with fewer remissions (about 50% of those with relapsing-remitting MS develop secondary progressive MS during the first ten years of their illness). Primary progressive MS : from the beginning, symptoms gradually develop and worsen over time (10-15% of people at onset). Acute attacks are followed by periods of remission when there is remyelination but, with advancing disease, this process begins to fail and periods

2008 Mentor

3045. Motor Neurone Disease

is not a normal feature but can affect some patients with bulbar palsy. Signs LMD dysfunction in the limbs manifests as weakness, atrophy, fasciculations and hyporeflexia. The thighs are often a site of marked fasciculation. Fasciculation can be difficult to distinguish from arterial pulsation, so consider if there is an underlying arterial course before defining twitching movements as fasciculation. UMN dysfunction manifests as weakness predominating in the arm extensors and leg flexors with evidence (...) of the disease is also called amyotrophic lateral sclerosis (ALS). It tends to be focal in onset, with a particular group of muscles affected first. This presents In three recognised patterns: Limb onset - by far the most common . Bulbar onset - 20% of cases . Respiratory onset - the least common . ALS: [ ] Presents with symptoms and signs of degeneration of the upper and lower motor neurons, leading to progressive weakness of the bulbar, limb, thoracic and abdominal muscles. Other brain functions, including

2008 Mentor

3046. Myopathies Full Text available with Trip Pro

affecting proximal muscle groups (shoulder and limb girdles) is typical. Weakness manifests itself in different ways at different ages: Decreased fetal movements in utero. Floppy infant neonatally. Motor delay in the toddler years. Reduced muscle strength and power in older children and adults. Myalgia may occur in inflammatory myopathies. Muscle-stretch reflexes are preserved. Somatosensory reflexes are preserved. Variation of strength with exercise (either increasing or decreasing) can occur (...) be associated including cardiomyopathy and conduction defects. Some metabolic myopathies: Hypokalaemia: oral supplements, cautious use of intravenous potassium, and prophylactic drugs (spironolactone and acetazolamide). Hyperkalaemia: carbohydrate loading (for example, early in attacks with hyperkalaemic periodic paralysis), glucose and insulin. : causes life-threatening renal complications and associated metabolic problems (hyperkalaemia). Usually requires intensive care management. Polymyalgia rheumatica

2008 Mentor

3047. Cerebellar Signs including Cerebellar Ataxia

hypoplasia, , . . There are various other uncommon neurological or metabolic diseases which may involve the cerebellum. Idiopathic cerebellar ataxia - a diagnosis of exclusion. Presentation As the cerebellum is associated with motor control, lesions produce a range of movement disorders (ataxias). These can be differentiated by their time course. Lesions of the midline vermis of the cerebellum cause truncal ataxia, while lesions of the cerebellar hemispheres cause limb ataxia of the ipsilateral side (...) , and linguistic abilities, with affective disturbance ranging from emotional blunting and depression, to disinhibition and psychotic features. Examination Check eye movement - looking for ophthalmoplegia or nystagmus. Check fundi for papilloedema. Get the patient to stick his/her tongue out and move it from side to side (movement slowed). Ask the patient to repeat "baby hippopotamus" - look for dysarthria and abnormal speech rhythm and syllable emphasis. Examine arms for limb ataxia (see above): rebound

2008 Mentor

3048. Chorea

. The arms, legs, neck and tongue may also be affected. Causes include asphyxia, neonatal jaundice, Huntington's chorea, cerebrovascular disease and trauma. Management can be difficult but treatment options include medications (eg, diazepam, haloperidol, tetrabenazine), surgery and retraining techniques. Chorea Continuous jerky movements in which each movement is sudden and the resulting posture is held for a few seconds. This usually affects the head, face or limbs. The focus may move from one part (...) in a relaxed limb when fully supported at rest. Causes include Parkinson's disease, Parkinsonism, other extrapyramidal diseases, multiple sclerosis. Postural - occurs if a limb is static (can also remain during movement). Types include physiological tremor, exaggerated physiological tremor - eg, in thyrotoxicosis, anxiety states, alcohol abuse, drugs (eg, sympathomimetics, antidepressants, valproate, lithium), heavy metal poisoning ('hatter's shakes' from mercury). Neurological disease - eg, severe

2008 Mentor

3049. Restless Legs Syndrome

cannot be accounted for as symptoms primary to another medical or a behavioural condition. Examples given in the criteria are myalgia, venous stasis, leg oedema, arthritis, leg cramps, positional discomfort, or habitual foot tapping. Supportive criteria: Positive response to dopaminergic treatment. Periodic limb movements during wakefulness or sleep. Positive family history of RLS amongst first-degree relatives. Lack of profound daytime sleepiness. Clinical course may be: Chronic-persistent (...) with iron deficiency. Renal function: RLS may be associated with end-stage chronic kidney disease. Other investigations for underlying possible cause include fasting blood glucose, magnesium, TSH, vitamin B12 and folate. If the neurological examination suggests an associated peripheral neuropathy or radiculopathy, electromyography and nerve conduction studies should be undertaken. Associated disorders Periodic limb movement disorder (PLMD) [ ] PLMD is a disorder characterised by periodic episodes

2008 Mentor

3050. Rheumatic Fever

important. In hot countries, skin infection is a more important source of streptococci than pharyngitis. Young age is a risk factor [ ] . Presentation [ ] Symptoms appear between one and five weeks after a sore throat, with an average of three weeks. In recurrent cases this incubation period is shorter, in keeping with a faster immune response. The diagnosis is based on major and minor criteria. In the acute disease the arthritis and toxicity are obvious but it can be more insidious with mild carditis (...) infection (eg, history of scarlet fever, positive throat swab or rising or increased antistreptolysin O titre (ASOT) >200 U/mL or DNase B titre). Plus two major criteria; or One major and two minor criteria. Definitions of these criteria are set out below. Major criteria Arthritis: The most obvious presenting feature is a flitting or migratory arthritis affecting large joints like the knees, ankles, wrists and elbows. The joints are hot and red with decreased range of movement. Typically, one joint

2008 Mentor

3051. Rett Syndrome

as a consequence of altered autonomic control. Secondary vascular changes in the long-term lead to abiotrophic changes in the lower limb and feet. Stage 3 - stationary or pseudostationary phase Typically aged 2-10 years. There may be an improvement in behaviour, use of the hands and communication skills. Eye contact returns and non-verbal communication may be exploited. There is persisting intellectual impairment with stereotyped hand movements. Generalised rigidity, bruxism and movements of the tongue may (...) deterioration or regression Typically age 1-4 years. May be a sudden onset of deterioration with an identifiable day when things changed; however, it may be a more subtle onset and progression in some cases. Early growth restriction may be noted in falling off of head circumference from growth curve. There are autism-like behaviours with loss of verbal and other communication, hand use and social interaction. There are abnormalities of hand movements when the patient is awake, with the hands usually held

2008 Mentor

3052. Blepharospasm

find one of our more useful. In this article In This Article Blepharospasm In this article The normal adult blinks at a rate of 10 to 20 times per minute. This tends to be reduced when reading or using the computer. An increase in lid closure frequency and tone is known as blepharospasm. This is a focal dystonia appearing in adults with recurrent spasms of eye closure; the orbicularis oculi muscle contracts forcibly and involuntarily. This lasts for periods varying from seconds to minutes and often (...) to a failure of contraction of the levator palpebrae muscle, which is the case in apraxia of eyelid opening, which occurs in Parkinsonian conditions. The aetiology of blepharospasm is not understood. It has been thought to be caused by pathology within the basal ganglia, although this has not been proven. It appears that multiple cortical and subcortical structures may be involved. [ ] The circuit involved in blinking involves a sensory limb, a central control area in the midbrain and a motor limb

2008 Mentor

3053. Behçet's Disease

systems. In many patients the activity of this disease diminishes with time. The International Classification Criteria of Behçet's Disease are as follows: [ ] In the absence of other clinical explanations, patients must have recurrent oral ulceration (aphthous or herpetiform) observed by the physician or patient, recurring at least three times in a 12-month period, and two of the following: Recurrent genital ulceration. Eye lesions: anterior uveitis, posterior uveitis, cells in the vitreous by slit (...) of patients have uveitis. Other ocular manifestations include hypopyon, retinal vasculitis, retinal haemorrhage, blurred vision and photophobia. Neurological: central nervous system (CNS) involvement occurs late in the disease. Memory impairment is the most common CNS manifestation. Impairment of balance, speech and movement can also occur. Thrombosis within the dural venous sinuses may occur. Peripheral nerve involvement is rare. [ ] Cardiovascular: Vascular lesions include vasculitis of the small

2008 Mentor

3054. Acute Abdomen

(including whether new pain or previously experienced). Site (ask the patient to point), localised or diffuse. Nature (constant/intermittent/colicky). Radiation. Severity. Relieving/aggravating factors (eg, if worsened by movement/coughing, suspect active peritonitis; pancreatitis is relieved by sitting forward). Associated symptoms: Vomiting and the nature of vomitus (undigested food or bile suggests upper GI pathology or obstruction; faeculent vomiting suggests lower GI obstruction). Haematemesis (...) period. History of sexually transmitted infections/pelvic inflammatory disease. Previous gynaecological or tubal surgery. Previous ectopic pregnancy. Vaginal bleeding. Drug history and allergies - including any complementary medication. Examination See also the separate article on . Pulse, temperature and blood pressure. Assess respiratory rate and pattern. Patients with peritonitis may take shallow, rapid breaths to reduce pain. If there is altered consciousness, check Glasgow Coma Scale (GCS

2008 Mentor

3055. Acromioclavicular Joint Problems

and/or localised AC joint pain. May be simply an ache in the deltoid area. Often worse at night. Local tenderness with exacerbation on passive and active shoulder movements. Cross-adduction (ie reach over front of opposite shoulder) often worsens pain; further passive cross-adduction by the examiner may aggravate this. It is important to differentiate OA from instability. Reduced range of movement is rare unless there is a prolonged period of arthritis. Differential diagnosis Clavicular fracture. Shoulder (...) on research evidence, UK and European Guidelines. You may find the article more useful, or one of our other . In this article In This Article Acromioclavicular Joint Problems In this article Synonyms: collar bone/clavicle disorders Description The acromioclavicular (AC) joint is the articulation between the acromion process of the scapula and the lateral end of the clavicle. It is one of the important functional joints that allows a full range of movement at the glenohumeral joint. A joint capsule

2008 Mentor

3056. Abnormal Gait Full Text available with Trip Pro

. The related affect may seem inappropriately unperturbed, or unusually hostile to investigatory questions. Choreic gait Wide-based gait, with slow leg raising and simultaneous knee flexion. Associated with choreoathetoid movements of the upper limbs. Causes include Huntington's chorea and dopaminergic medication. Medication-related gait disturbance [ ] Medications are a factor in at least 30% of the elderly with balance or gait problems. Polypharmacy (more than four medications) is a risk factor for falls (...) . You may find one of our more useful. In this article In This Article Abnormal Gait In this article Balance is the ability to stand, and gait is rhythmic stepping movements for travel (locomotion). Balance and gait problems tend to be found in the same individuals. Walking messages are initiated by the motor and premotor cortex and modified by the subcortical nuclei, brainstem, and cerebellum. These all activate the spine's central pattern generator, which coordinates arm and leg movements

2008 Mentor

3057. Abbreviations

PIVKA-II protein induced from vitamin K absence PJRT permanent junctional re-entrant tachycardia PLDH pegylated liposomal doxorubicin hydrochloride PLHA people living with HIV/AIDS PLMS periodic limb movements of sleep PLP1 proteolipid protein-1 PLS primary lateral sclerosis PM polymyositis PMA progressive muscular atrophy PMETB Postgraduate Medical Education Training Board PML progressive multifocal leukoencephalopathy PMN polymorphonuclear PMR polymyalgia rheumatica PND postnasal drip po per os (...) LLETZ large loop excision of the transformation zone LMA laryngeal mask airway LMP last menstrual period LMWH low molecular weight heparin LOC loss of consciousness LOD laparoscopic ovarian 'drilling' LSCS lower segment Caesarean section LSD lysergic acid diethylamide LTK laser thermal keratoplasty μg (mcg) microgram m metre m3 cubic metre mm3 cubic millimetre mm Hg millimetre of mercury mol mole m/r modified-release MAC membrane attack complex MALT mucosa-associated lymphoid tissue MAOIs monoamine

2008 Mentor

3058. Tremor

. Arsenic, heavy metal, organophosphate or industrial solvent poisoning. Vitamin deficiency (especially B1). Classification of tremors Tremors can be initially classified as rest or action tremors. [ ] Rest tremors occur when the body part is supported against gravity - eg, hands at rest in one's lap. Mental stress or general movement makes rest tremors worse. Action tremors are further subdivided into static, postural or kinetic tremors: Static - occurs in a relaxed limb when fully supported at rest (...) movement or sensation. Symptoms Essential tremor (ET) : This is usually a distal symmetrical postural tremor of the upper limbs, usually of low amplitude with a fairly rapid frequency of 8-10 Hz. It may initially be transient but usually progresses to become persistent. The neck muscles may be involved, causing tremor of the head (about 40% of cases). Voice, face and jaw muscles may be involved. [ ] Frequency of the tremor tends to remain constant but amplitude is highly variable depending on emotional

2008 Mentor

3059. Whiplash and Cervical Spine Injury

injury occur as the result of rear-end vehicle collisions at speeds of less than 14 miles per hour. Patients present with neck pain and stiffness, occipital headache, thoracic back pain and/or lumbar back pain and upper-limb pain and paraesthesia. [ ] There are two types of injury: Typical cervical hyperextension injuries occur in drivers/passengers of a stationary or slow-moving vehicle that is struck from behind. The person's body is thrown forward but the head lags, resulting in hyperextension (...) of the neck. When the head and neck have reached maximum extension, the neck then snaps into flexion. A rapid deceleration injury throws the head forwards and flexes the cervical spine. The chin limits forward flexion but the forward movement may be sufficient to cause longitudinal distraction and neurological damage. Hyperextension may occur in the subsequent recoil. Whiplash-associated disorders (WAD) can be classified by the severity of signs and symptoms: [ ] Grade 0: no complaints or physical signs

2008 Mentor

3060. Werdnig-Hoffmann Disease

than distal, with lower limbs generally weaker than upper limbs. Deep tendon reflexes are absent or diminished but sensitivity is preserved. In the most severe forms decreased intrauterine movements suggest prenatal onset of the disease and present with severe weakness and joint contractures at birth (this has been labeled SMN0). Some of these children may also show congenital bone fractures and extremely thin ribs. The spared diaphragm, combined with weakened intercostal muscles, results (...) . Within SMA1 at least three clinical subgroups can be defined according to the severity of clinical signs: Severe weakness since birth/neonatal period - head control is never achieved. Onset of weakness after the neonatal period but generally within two months - head control is never achieved. Onset of weakness after the neonatal period but head control is achieved. Some of these children may be able to sit with support. Differential diagnosis Includes: Amyotrophic lateral sclerosis. Congenital

2008 Mentor

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